Portuguese Version

Year:  2002  Vol. 68   Ed. 5 - (26º)

Relato de Caso

Pages: 767 to 769

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Juvenile xanthogranuloma of the nasal cavity

Author(s): Melissa GA Avelino 1,
Fabiana C Pereira 1,
Kátia XR Moreira 2,
Reginaldo R Fujita 3,
Aldo EC Stamm 4,
Luc LM Weckx 5

Keywords: juveline xanthogranuloma, nasal cavity, tumor, immunohistochemic, histiocytosis

Abstract:
Juvenile Xanthogranuloma is a benign disease, classified as Class II histiocytosis, generally characterised by cutaneous nodules, most commonly, in the head and neck region. It often occurs in childhood and it is rare in adults. Extracutaneous lesions are uncommon, and the most common extracutaneous lesion is in the eye. We report a case of juvenile xanthogranuloma occuring in the nasal cavity, which is extremely rare in literature. We found only one report of xanthogranuloma in nasal cavity in the English Literature1. Case report: D.C.S., female, 12 years-old was referred with a history of progressive right sided nasal obstruction for 18 months. In another hospital, she had had diagnostic of Rhinoscleroma. The clinical examination revealed a solid lesion in the right nasal cavity with an increased dorsal nose and deslocation of right eye. The solid lesion was excised by Degloving technique. The histopathological and immunohistochemical diagnosis was of juvenile xanthogranuloma. Juvenile Xanthogranuloma is a benign disease which regresses spontaneously. We observed the patient with nasofibroscopy for five months after surgery. Until today, she is asymptomatic and without recurrence.

Introduction

Juvenile xanthogranuloma (JXG) is a benign disease that affects children, characterized by single or multiple cutaneous nodules. The lesions are commonly found in the head and neck region. The most affected extracutaneous site is the eye2. Other involved sites are oral cavity (tongue, soft palate, gums, tonsil pillars), nasopharynx, salivary glands, hard palate, spleen, liver, lung, pericardium, ovary and testis.

Single lesion manifestations are detected in 82% of the cases. The incidence is still unknown and a male tendency has been noticed1, 2. JXG is a class II histiocytosis tumor (non-Langerhans cells). It is predominant in childhood and regresses spontaneously.

The case we report below is a juvenile xanthogranuloma of the nasal cavity, extremely rare and seldom described in the literature.

Literature Review

Juvenile xanthogranuloma (JXG) was first reported in the literature in 1905 by Adamson3 who called it multiple congenital xanthoma. McDonagh4 described a new case in 1909 and other five cases in 1912. Only in 1954 Helwig and Hackney5 proposed the name juvenile xanthogranuloma.
In 1993, Caputo6 reported a case of congenital giant JXG of the nose and Saravanappa1 in July 2000 reported a case of nasal cavity JXG, which was the first nasal cavity JXG of the literature in English. There are, therefore, very few references to nasal cavity JXG in the literature.

CASE REPORT

DCS, 12-year-old female patient, born and living in São Paulo, presented history of progressive nasal obstruction on the right for 1 year and a half. At the physical examination, we observed occlusion of the right nasal fossa by a solid tumor, non-bleeding upon touch and that caused bulging of the nasal pyramid and slight deviation of the orbit. Two biopsies were conducted in another service: the first one was not conclusive and the second one was suggestive of rhinoscleroma.

The patient was submitted to exeresis of the tumor using the mid-facial degloving technique. During the surgery, we observed that the tumor had a necrotic/soft aspect and occupied the right nasal fossa and also the right maxillary and ethmoid sinuses, including impairment of the periorbital fat. The surgical specimen was sent to anatomical pathology and immunohistochemistry analyses. At microscopy, we observed histiocytes, giant cells, lymphocytes and eosinophils, and at immunohistochemistry, CD68-positive, S100-negative, HHF35-positive, concluding that it was a juvenile xanthogranuloma.

After the diagnosis, the patient was referred to ophthalmologic assessment, but no ocular involvement was detected. She did not present any type of skin compromise and on the 5th postoperative month there was no apparent recurrence. She has been submitted to monthly follow-up with nasofibroscopy.

Discussion

Juvenile xanthogranuloma is the most common form of non-Langerhans cells histiocytosis or non-X histiocytosis and its real incidence is unknown7. It normally occurs during childhood, frequently manifests as a solitary lesion that regresses spontaneously as years go by. In the case reported here, the patient was 12 years old and the lesion was single, but it had caused facial abnormalities and significant nasal obstruction. It was necessary to remove it, for diagnosis and esthetical and functional correction. In the literature, there is a slight predominance of male patients, of 1.5: 18, even though our patient was female. It may affect all races, but there are few reports of the disease in black patients5. Even though it is rarer in black patients, our patient was daughter of black people.

The most common clinical manifestation is cutaneous impairment, through the nodules that vary from 0.5 to 2.0cm in diameter, especially in the head and neck region. At initial stages, cutaneous lesions present pink-reddish color, acquiring later a brown-yellowish aspect, which seems to be like telangiectasis on the surface2. Single lesions are found in 60 to 82% of the patients8, 9, 10. Extracutaneous affection is more common in the eyes, with an incidence of 0.3 to 0.5% in patients with cutaneous JXG. In 41% of the patients with ocular involvement, the cutaneous lesions are multiple. The ocular involvement is more frequent in the first two years of life11.

Cohen2 reported affection of the oral mucosa by JXG. Martin7 reported various sites of the lesion, such as lung, testis, ovaries, pericardium, spleen, bone, retroperitonium, CNS, larynx, adrenal gland, among others. The patient did not present any other type of cutaneous lesion and had no other extracutaneous manifestations. The lesion was single and in the nasal cavity, which is very rare according to literature findings.

JXG is microscopically characterized by the presence of histiocytes, giant cells and inflammatory cells10. Immunohistochemical study demonstrates that xanthogranuloma is positive to XIIIa factor, KP1, KiM1P, HAM 56 and HHF35; and negative to S100 protein and MAC38712. In the clinical case reported here, the immunohistochemical study enabled our diagnostic conclusion.

In the differential diagnosis we should consider a series of disorders that present similar chemical and histopathology findings, such as X histiocytosis. In this affection, we should investigate positive S-100, whereas JXG S100 is negative. Our patient had HHF35 positive and S100 negative. Malignant tumors, such as rhabdomyosarcoma, fibrosarcoma and malignant fibrohistiocytoma, should be remembered for the differential diagnosis. The immunohistochemical findings help the definition of the correct diagnosis in atypical manifestations of JXG and to differentiate it from other X and non-X histiocytosis, which happened in our case.

There two important associated conditions with JXG 1, 3, 13: the first one described by Newell in 1973 is neurofibromatosis, the second one described by Cooper in 1984 and by Zvulonov in 1995 is chronic myeloid leukemia, which were not found in our patient.

The treatment for juvenile xanthogranuloma is observation, because regression is spontaneous, requiring surgical intervention if there is esthetical affection. Ocular lesions can be treated with surgery, radiotherapy and corticoids14.

closing remarks

JXG normally does not require any type of treatment when the lesions are asymptomatic. Surgical treatment for exeresis of the lesion is performed for diagnosis or when there is esthetical impairment, as in the case described here.

REFERENCES

1. Saravanappa N et al. Juvenile xanthogranuloma of the nasal cavity. The Journal of Laryngology & Otology 2000 June;114:460-461.
2. Cohen BA, Hood A. Xanthogranuloma: report on clinical and histological findings in 64 patients. Pediatr Dermatol 1989;6:262-6.
3. Adamson NF. Congenital xanthoma multiplex in a child. Br J Dermatol 1909; 21:254.
4. McDonagh JER. A contribution to our knowledge of naevoxanthoendothelioma. Br J Dermatol 1912;24:85-9.
5. Helwing EB et al. Juvenile xanthogranuloma. Am J Pathol 1954;30:625-6.
6. Caputo R et al. Unusual aspects of juvenile xanthogranuloma. J Am Acad Dermatol 1993;29:868-70.
7. Martin AH et al. Juvenile xanthogranuloma. Journal of the American Academy of Dermatology 1997;36:355-65.
8. Yanof M et al. Juvenile xanthogranuloma of the corneoscleral limbus. Arch Ophthalmol 1995;113:915-7.
9. Sonoda T et al. Juvenile xanthogranuloma. Cancer 1985;56:2280-6.
10. Tahan SR et al. Juvenile xanthogranuloma. Arch Pathol Lab Med 1989;113:1057-61.
11. Zirmmerman LE et al. Ocular lesions of juvenile xanthogranuloma. Trans Am Acad Ophthalmol Otolaryngol 1965;69:412-42.
12. Zelger B et al. Juvenile and adult xanthogranuloma: a histological and immunohistochemical comparison. Am J Surg Pathol 1994;18:126-35.
13. Zvulonov A et al. Juvenile xanthogranuloma, neurofibromatosis and juvenile chronic myelogenous leukemia: world statistical analysis. Arch Dermatol 1995;131:904-8.
14. Casteels I et al. Early treatment of juvenile xanthogranuloma of the iris with subconjuctival steroids. Br J Ophthalmol 1993;77:57-60.

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1 Specialization under course, Discipline of Pediatric Otorhinolaryngology, UNIFESP-EPM.
2 Post-graduation under course, Discipline of Pediatric Otorhinolaryngology, UNIFESP-EPM.
3 Clinical Head of the Discipline of Pediatric Otorhinolaryngology, UNIFESP-EPM.
4 Affiliated Professor, Discipline of Pediatric Otorhinolaryngology, UNIFESP-EPM.
5 Head of the Discipline of Pediatric Otorhinolaryngology, UNIFESP-EPM.

Discipline of Pediatric Otorhinolaryngology, UNIFESP - EPM.

Address correspondence to: Rua dos Otonis, 674/684 - Vila Clementino - São Paulo - SP - Tel: (55 11) 5539.7723

Article submitted on July 20, 2001. Article accepted on August 08, 2001

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